Cases reported "Facies"

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1/8. Partial anodontia in a case of Waardenburg's syndrome.

    On routine examination of a case of Waardenburg's syndrome apart from the usual features of the disorder, partial anodontia involving both the lower lateral incisors was evident. The interesting association of partial anodontia and Waardenburg's syndrome is discussed along with relevant available literature.
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keywords = anodontia
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2/8. The KBG syndrome, characteristic dental findings: a case report.

    Short stature and developmental delay may be observed in many genetic conditions and well-defined syndromes. A 7-year-old girl presented with the non-specific findings of subtle dysmorphism, short stature and developmental delay. Although a genetic syndrome was suspected, a definitive diagnosis was not made until the dental findings of KBG syndrome were recognised, namely grossly enlarged maxillary permanent central incisors and hypodontia.
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ranking = 0.0026800123734064
keywords = hypodontia
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3/8. Hypohidrotic ectodermal dysplasia: dental, clinical, genetic and dermatoglyphic findings of three cases.

    patients with hypohidrotic ectodermal dysplasia (HED) are characterized by the clinical manifestations of hypodontia, hypohidrosis, hypotrichosis and a highly characteristic facial physiognomy. This disorder is inherited as an X-linked trait. This report presents three cases with HED in which the clinical evaluation (intraoral and radiological), genetic findings and SEM examination of hair. Boys 6 to 14 year old and a 11 year old girl were referred to the Marmara University, faculty of Dentistry, complaining of oligodontia in the maxillary and mandibular arches and delay in eruption of other teeth. Peg-shaped teeth have been observed. The dermatoglyphs of the patients were striking. SEM examination of hair demonstrated a distinctly abnormal longitudinal grooving along the entire length of each hair and a desquamation of the surface cuticles. The treatment was planned in a multidisciplinary odontological group involving pediatric dentistry, orthodontics, prosthodontics and oral surgery and maxillofacial radiology of future dental habilitation. A specially designed overdenture, a removable prosthesis and osseointegrated implants were constructed. Periodic recall visits were advised, to monitor the dentures and implants during periods of growth and development, and eruption of the permanent teeth.
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ranking = 0.0026800123734064
keywords = hypodontia
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4/8. Clinical management of hypohidrotic ectodermal dysplasia with anodontia: case report.

    ectodermal dysplasia is a rare hereditary disorder with a characteristic physiognomy. The case of a 5-year-old child with hypohidrotic ectodermal dysplasia and complete anodontia is presented. Because of the anodontia and the need for treatment at an early age, the prosthetic management of such a young child can be difficult. Complete dentures were provided to encourage a normal psychological development and to improve the function of the stomatognathic system.
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ranking = 1
keywords = anodontia
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5/8. The Kabuki syndrome: four patients with oral abnormalities.

    The aim of this paper is to report the oral signs and symptoms of four patients with Kabuki syndrome. All had oral abnormalities affecting the palate, teeth, and/or lips, including wide spacing between the teeth, screwdriver-shaped upper incisors, hypodontia, delayed tooth eruption, narrow spacing in the upper canine area, large pulp chambers of the upper incisors and permanent molars, external root resorption of the upper central incisors and permanent molars, a division of the lower third of the root canal in normally one-rooted teeth, tooth retention, retrognathia of the upper jaw, a high palate or cleft lip/palate, and microforms of lower lip fistula.
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ranking = 0.0026800123734064
keywords = hypodontia
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6/8. Dental anomalies in Axenfeld-Rieger syndrome.

    The authors describe the case of a 10-year-old girl presenting with Axenfeld-Rieger syndrome (ARS), a rare autosomal dominant condition. The patient showed severe hypodontia, microdontia and short roots. early diagnosis of the syndrome from its dento-facial and systemic features is important so that subsequent ocular complications may be prevented.
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ranking = 0.0026800123734064
keywords = hypodontia
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7/8. Dental findings of a child with wolf-hirschhorn syndrome.

    The case presented is that of a 5-year-old female with wolf-hirschhorn syndrome. Dental findings were severe hypodontia, late dental development, taurodontism of the primary molars, microdontia, and spacing. Hypodontia has previously been reported and therefore this case adds to the evidence that hypodontia may be a common feature of this syndrome. It also suggests that other dental anomalies could occur in children with wolf-hirschhorn syndrome.
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ranking = 0.0053600247468127
keywords = hypodontia
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8/8. Distraction in a case of otopalatodigital syndrome type II.

    INTRODUCTION: Otopalatodigital syndrome type II is a rare X-linked recessive disorder with generalized skeletal dysplasia and hearing anomalies. Its features include conductive hearing loss, unusual facies, cleft palate, micrognathia, and overlapping flexed fingers and toes. It is a more lethal variant of otopalatodigital syndrome type I. There are many consistently reported craniofacial and dental findings; however, no case has been published in dental literature. CASE REPORT: We report a case of otopalatodigital syndrome type II with micrognathia, cleft of the soft palate, and partial anodontia, and discuss the combined orthodontic and surgical management. CONCLUSION: We also discuss the differential diagnosis and consider more recent theories on possible aetiology as well as clinical management strategies for such cases.
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ranking = 0.16666666666667
keywords = anodontia
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